The Scleroderma Chronicles: Upsetting vs. Concerning

I haven’t been blogging all that much about my chronic conditions lately… the truth is, I hardly know what to say. I’m sick. I still struggle every day. I’ve been making a lot of adjustments over the last couple of years as I tackle never-ending obstacles, but I really don’t dwell on things too much and I don’t make too many demands of my doctors. I’m still happy, busy, and I manage work-arounds for lots of things that I want to do. Then suddenly this winter I realized that I wasn’t doing all that well after all.

Background: For several years I have been consistently complaining to my doctors that I am short of breath, fatigued, itching, rocking blue lips, and not quite right. My doctors are all like… hmm… that is interesting. Well, it isn’t (insert specific issue that this doctor treats me for), so I’m not sure what to do. We’ll keep monitoring… Maybe you should ask this other doctor about it…

This last December I really hit the wall. I was so exhausted that I was afraid to go shopping by myself. I ended up on the floor panting for air more than once. I collapsed in the yarn store. My lips were blue. The itching drove me crazy. Something in my side hurt and strange bruising kept popping up. Fainting suddenly became an issue again.

I spent most of December in bed reading books and knitting gnomes!

I went to my internist and told her what was up and she immediately ordered several tests. Some scary things like lymphoma were ruled out, but others suddenly reared their ugly heads. I have way too many red blood cells and too much hemoglobin. My cell count spiked to its highest last December when my symptoms were at their worst. Suddenly a new diagnosis was placed on my chart: polycythemia.

Now the fun really begins. I have a lot of things written on my chart. I am diagnosed with several autoimmune conditions: limited systemic sclerosis  (scleroderma), Sjogren’s Disease, and fibromyalgia. These diseases come with a boat load of complications as they tend to cause trouble everywhere they go: I have stage 3 kidney disease, lung disease, hypertension, Raynaud’s Phenomenon, gastroparesis, tendon damage, bursitis, several crazy eye issues, fluid around my heart, and a stomach that has herniated partly into my chest. Seriously, who knew that was a thing? Actually, my whole GI tract is in trouble. My muscles hurt and the joints are swollen. Do you see how confusing treating me can be when a new medical problem emerges? No one wants to rock the boat! My diagnosed conditions actually make it harder for me to get good health care at this point. I know that I am a high risk patient, but this is ridiculous, doctor people!

My internist checked to see if I had the very serious and rare version of polycythemia called polycythemia vera, and since my hormone levels were normal she concluded that my polycythemia was being caused by an underlying condition, and we should just monitor my red blood cell count in ongoing routine rheumatology blood work.

What? Wait… what about my blue lips, panting and all the other symptoms? My immediate reaction was… OH, NO, WE ARE NOT DOING THAT!!! I am done with the ending up on the floor panting every time I try to do something reasonable like… say… cooking dinner! I dusted off the biological researcher part of my brain, generated a list of essential questions, and did a bunch of online searches to see what I could find. The trick to this is to have good questions and the right seach terms. As I worked, things became more productive. Wow. I learned a lot and now I had more questions for my internist. I wrote her back an email with a bulleted list of questions that… she never got. She’s on vacation. One of her partners looked at the email, became alarmed by my symptoms, and called to urge me to head to an ER immediately (!!) to be evaluated. She was pretty insistent…

Which kind of proves my point! Anyone else who ended up on the floor with blue lips, panting for air, because she tried to vacuum the living room would receive timely medical intervention!! This is not reasonable. I’m not getting the care that I should because I am too complicated and there are too many doctors involved. This has been going on for three years and now it is worse. Did one of your parents ever say to you when you were a kid misbehaving in the backseat, “Don’t make me pull this car over!” I am now pulling the car over and stopping this ride.

This is kind of intense, huh. Let’s take a break to admire my favorite orchid!

Tonight as I write this I am just hours away from a phone appointment with my internist. I have my questions ready to go. In the afternoon I have another phone appointment with my pulmonologist; I plan to tell her about the new diagnosis and to ask her about testing that I should have to help identify the underlying cause of the polycythemia. Maybe she will have some other ideas. Tuesday morning I have an appointment with my new rheumatologist and I hope to pass all of this to her. This rheumatologist specializes in complicated cases, is recommended by the Scleroderma Foundation, and prefers to personally coordinate the entire care team. Yay! Here I come! I am the girl for her!

There are many things that are happening to me that are upsetting: my hair is falling out, I have a rash on my face, and I now use a cane to get around. This is the kind of stuff that makes you want to sigh and stay in bed some mornings. You wish it wasn’t happening to you. It’s upsetting.

Then there are things that are just down right concerning. In my mind a red flag pops up and a small siren goes off: strange and unexplained bruising, double vision, pain in my left side, blue lips, panting for air while trying to open a door, tremendous itching after showers. This kind of stuff is a call to action; get out of bed and deal with it! I am now mobilizing all of my energy towards GETTING TO THE BOTTOM OF THIS and securing answers, treatment, and hopefully, an improved quality of life.

Because there is a difference between upsetting and concerning. I am not upset, but I am concerned. I am mobilized. I have questions and I want answers.

I am totally over the blue lips look!

I’ll let you know how this all works out. It should be a busy week.

The Scleroderma Chronicles: Year 3.0

My, how the time flies. Not that I’m having a good time here, but it is hard to believe that it has already been three years since my diagnosis of limited systemic sclerosis (AKA scleroderma). I’ve been reflecting on the last year while planning this post, and decided that I should start out with a little info about my disease, share the highlights of my three years, and then give unpack this year a little.

Butterfly
But first, a butterfly picture! Butterflies have hard skin, and they are doing OK. Be like a butterfly, I tell myself. This little lady was part of a massive migration of butterflies that came through our state in the fall. Seriously, there were so many of them that they showed up on the weather radar and the NWS put out a bulletin asking the public to identify the bird species: not birds, but butterflies.

Scleroderma is an autoimmune disease that is chronic (no cure), progressive, disabling and possibly fatal. It is rare, which is why you probably never heard of it. The name itself means “hard skin”, and that is one of the most distinctive features of the condition. The widespread scarring and buildup of collagen protein that causes the hardening of skin also occurs in internal organs in patients with the “systemic” form of the disease like me. Most of the damage is hitting my intestinal tract, but my lungs, kidneys and heart are also sustaining damage. In the background, hard to see, but never to be ignored, is damage occurring in blood vessels that can cause blood pressure to soar and places me at risk for blood clots.

I have collected several doctors over the last three years as damage continues to slowly accrue in my lungs, kidneys and intestinal tract.

Here are the highlights of my first three years:

  1. My first year was one of shock and horror. I was so worried about tightening skin and the use of my hands that I didn’t ever think about the bigger picture. I was started on drugs, stabilized, and felt much better by the end of the year.
  2. The bottom fell out my second year. I was using my hands okay, but I developed breathing problems, had to be placed on oxygen, and my heart started to misbehave. Adjustments were made to my medications to compensate for my lowering lung volume and to slow the rate of lung damage. At the lowest point I was sent to palliative care and told to make final decisions.
  3. This year, the third, has been one of highs and lows. The new medications kicked in, I came off oxygen and my chest pain stopped. I was discharged from palliative care. I developed gastroparesis and had to move to a very stomach-friendly diet. I stabilized and sailed through the first rounds of appointments in the spring only to develop kidney problems in the summer along with higher pressure in the artery that goes from my heart to my lungs. This blood pressure, which is called pulmonary arterial hypertension, is extremely damaging to the heart and will need to be addressed if it gets any higher. Fabulous. Another doctor.

My summer this year was really hard. I got very dizzy, developed joint pain and sore muscles, and eventually got so brain fogged that I was afraid to drive. My knees were swollen and developed sharp, shooting pains; I will need to buy a new car if this keeps up as I can’t manage the clutch much longer. I struggled on the stairs and my face turned blue on a regular basis. My neighbors stepped in and took over the yard work for me, and my knitting buddies began to drive me to all fiber related adventures as I wasn’t sure I should be trusted on the road. See, highs and lows. My illness is kicking my butt, and the people around me are stepping in to make sure I’m OK.

Early this month I met with my new internist to see if there wasn’t something that I could take to beat some of these symptoms back. OK, I’m going to be honest here. I cried. We agreed that I would start the tart cherry extract again, but at a much lower dose than I took previously in the spring. (Tart cherry has anti-inflammatory properties and is easier on my stomach than NSAIDs. Unfortunately, my kidneys were damaged the first time I tried to take it.) I’m going to have my kidney function checked every month, but I’m already so much better (brain fog, goodbye!) that I’m really hoping that I can tolerate it OK. In the meantime I’m getting lots of chores done in this golden period while I feel so much better. I’ve moved furniture, completed some projects, and have driven to many, many stores that were ignored last summer.

Wizard Hat
And I made a crocheted had for my niece to wear this Halloween. Really, I’ve been a bundle of energy the last couple of weeks.

So, this is the end of the third year. I feel pretty good, I’m getting things done and making plans, and I am making hay while the (tart cherry) sun shines. Next week I get my blood drawn for the kidney function test and after that I see the rheumatologist.

I’ve been thinking about butterflies again. The day after I took that picture of the butterfly it snowed. A lot.  It took a couple of days for it to melt as the temperatures climbed back up into the 60’s and 70’s. I wondered if the butterflies would make it. As I walked out of the office building after seeing my internist (and still recovering from my crying fit in her office…) I found butterflies swarming around one of the shrubs by the parking lot.

Those butterflies. You can kick them, but they come back. Be like a butterfly, I tell myself.

And the fourth year begins.